Alternate Names: Tumor - retina; Cancer - retina

Causes and Risks: The cause of most cases of retinoblastoma has recently been determined to be the absence of a tumor suppresser gene. Retinoblastoma and other associated malignancies tend to run in families.

One or both eyes may be affected. A visible whiteness in the pupil may be present. Blindness can occur in the affected eye, and the eyes may appear crossed. The tumor can spread to the eye socket, and to the brain, by means of the optic nerve. The risk factors other than a family history are not known. This is a rare tumor.

Prevention: Genetic counseling can help predict the risk of retinoblastoma if a family history is present.

Symptoms:

• A white glow in the eye that is often seen in photographs taken with a flash; instead of the typical "red eye" from the flash, the pupil may appear white or distorted
• Pupil, white spots
• Crossed eyes
• A red, painful eye
• Poor vision
• The iris may be a different color in each eye

Signs and Tests:

• An examination of the eye with dilation of the pupil
• A CT scan of the head to evaluate tumor and possible spread
• An ultrasound of the eye (head and eye echoencephalogram)

Treatment: It is important to seek treatment by a physician with experience treating this type of tumor. Treatment options depend upon the size and location of the tumor. Small tumors may be treated by laser surgery. Radiation and chemotherapy may be indicated if the tumor has spread beyond the eye. The eye may be removed if the tumor does not respond to therapy.

Prognosis: Relative to most cancers, the overall prognosis and chance for cure is good. There are times, however, that the function of the involved eye is lost or the eye must be removed in order to maximize the chance of a cure.

Complications: Spread of the tumor to the brain or other locations can occur.

Call your health care provider if signs or symptoms of retinoblastoma are present, especially if your child's eyes appear abnormal in photographs or when you look at the child.